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Section 5
Biliary tract
The biliary tract has an intra- and extrahepatic course. In most cases treatment
of intrahepatic bile duct pathology will be combined with a liver resection.
Extrahepatic bile duct disease may not involve any other structure but because
of the intimate relations may require an additional pancreatic resection. Not
infrequently, bile duct disease includes both the intra- and extrahepatic por-
tions of the duct and requires combined hepatic and ductal surgery.
Patients with suspected bile duct pathology frequently present initially with
abnormal liver function tests and with very few symptoms apart from itching
and general malaise. Investigation of these patients ( Fig. 5.1 ) not infrequently
demonstrates a non-surgical cause for their jaundice.
If a surgical cause is suspected, generally because of the demonstration of a
dilated duct, then further specific investigations will be required.
Benign conditions
Sclerosing cholangitis
Primary sclerosing pancreatitis is a chronic cholestatic liver disease of unknown
aetiology. It produces diffuse inflammation and fibrosis of the biliary tree
mainly affecting young men in their 30s. The exact worldwide prevalance is
unclear but it seems to affect 1.5/100 000 men and 0.5/100 000 women (Bambha
et al ., 2003 ; Chapman RW, 2003 ). Isolated intrahepatic sclerosing cholangitis
can be treated by hepatic resection (Cameron et al ., 1988 ; Ahrendt et al ., 1998 )
but diffuse widespread disease is extremely difficult to treat and ultimately
may require liver transplantation (Wiesner et al ., 1996 ). Extrahepatic scleros-
ing cholangitis requires bile duct excision to include all strictures and is recon-
structed with a Roux loop (see Section 1). The difficulty in the clinical setting
is to distinguish sclerosing cholangitis from a malignant process, particularly
multifocal cholangiocarcinoma, and with the increased incidence of cholangi-
ocarcinoma in these patients this can be remarkably difficult (Burak et al .,
2004 ). There is occasionally some indication from investigations such as
MRCP and ERCP, where typically obstruction above a malignant obstruction
leads to dilatation. With sclerosing cholangitis this does not happen due to
subclinical involvement of the rest of the biliary tract (Talwalkar et al ., 2004 ).
BILIARY TRACT
254
Fig. 5.1 Investigation of abnormal liver function tests.
Dilated CBD
Investigate (see
Section 5)
Fasting lipid
profile normal
Exclude parasitic
causes
Stop all
medication where
possible
Malignant/
infiltrative process
replacing
parenchyma
Investigate
aetiology
Cirrhosis
No evidence
cirrhosis
Fasting lipid
profile abnormal
Potential drug induced
cholestasis
• New medications
• Change of medication
CBD not dilated
on US
Cirrhosis
suspected on CT
Abnormal
LFTs
BENIGN CONDITIONS
255
CA 19.9 is not elevated in sclerosing cholangitis but its sensitivity for cholan-
giocarcinoma still leaves many patients without a firm diagnosis (Ramage
et al ., 1995 ). PET appears to be a promising modality particularly for those
patients with sclerosing cholangitis where the development of cholangiocarci-
noma is suspected (Keiding et al ., 1998 ). If complete surgical excision can be
achieved then this is the treatment of choice and the distinction may be irrel-
evant, as histology will ultimately determine the situation (Claessen et al .,
2008 ).
Primary duct stones
In the absence of a gallbladder or stones in a gallbladder, calculi in the duct are
likely to be primary bile duct stones (Madden, 1973 ; Saharia et al ., 1977 ).
Management is directed at gaining adequate drainage from the duct. This is
usually achieved by ERCP and sphincterotomy. If recurrence of stones occurs
despite this, then stenting and regular changes will provide drainage of the
duct and hopefully inhibit further stone formation. If this fails to control the
problem, surgical intervention will be required. Usually the duct is larger than
normal and the surgical strategy is also directed at improving drainage. The
bile duct needs to be resected from just below the confluence of the right and
left hepatic ducts to the level of the pancreas. A 70 cm Roux loop anastomosed
to the upper resection level using the technique described in Section 1, and the
lower end is oversewn.
Inflammatory strictures ( Fig. 5.2 )
This is a difficult and relatively infrequent condition that presents a diagnos-
tic dilemma and both malignant and inflammatory processes have to be
considered (Hadjis et al ., 1985 ; Standfield et al ., 1989 ). The possible causes
of an inflammatory stricture include gallstones, pancreatitis, chronic duode-
nal ulceration, sclerosing cholangitis, parasitic infections, any localized
abscess adjacent to the bile duct, and, in the Far East, recurrent pyogenic
cholangitis. Although all these processes have to be considered, the clinical
setting and simple first-line investigations generally allow the majority to be
excluded.
When no obvious cause is found, further investigations are essential to
exclude a malignancy and to try to prevent unnecessary surgery for a benign
condition. However, despite attempts to gain brushings at ERCP (Foutch
et al ., 1990 ; Kurzawinski et al ., 1992 ), or even direct biopsy at operation, it may
be necessary to proceed to surgical resection in order to determine the nature
of the lesion. For mid-third strictures this is a relatively straightforward surgical
procedure but upper-third strictures may require a Roux-en-Y anastomosed to
BILIARY TRACT
256
Fig. 5.2 Investigation and management of a biliary stricture.
Biliary stricture
No previous
surgery
CT
MRCP suggests
incidental
malignancy
Previous surgery
MRCP suggests
iatrogenic injury
Recent surgery
low grade
stricture
ERCP stent and
review
Surgical repair if
fit
Surgery not
recent: High
grade stricture
ERCP and stent if
unfit
Pancreatic mass
See management
of pancreatic
cancer (Section 1)
No pancreatic
mass
Management of
cholangiocarcinoma
(Section 5)
BENIGN CONDITIONS
257
the left and right hepatic ducts. For the lower-third tumours, a decision to
proceed to a pancreaticoduodenectomy should probably be avoided if possible
(and certainly without histological proof of malignancy).
Polyps
The location of bile duct polyps will dictate the approach. Infection or obstruc-
tion is the usual presentation, and in the extrahepatic portion, these can be
managed with a Roux-en-Y anastomosis to the bile duct. Intrahepatic polyps
may require liver resection.
Choledochal cysts ( Fig. 5.3 )
Choledochal cysts are classified on the basis of their site, extent, and the shape
of the cystic abnormality of the duct system (Kamisawa et al ., 2008 ). The term
choledochal cyst is the one that has been most frequently used in the past but
since it has been realized that the same pathology can arise anywhere in the
biliary tract, as opposed to just from the bile duct which this term implies, they
have been increasingly referred to as biliary cysts.
The first classification was produced in 1959 (Alonso-Ley et al ., 1959 ) but the
recognition that intrahepatic bile duct cysts (Caroli et al ., 1958 ; Dayton et al ., 1983 )
Fig. 5.3 Types of choledochal cyst. a) Normal biliary tract. b) Solitary fusiform.
c) Extra-hepatic supraduodenal diverticulum. d) Intraduodenal diverticulum
(choledochocele). e) Fusiform intra- and extrahepatic cysts. f) Intrahepatic cysts
(multiple in Caroli’s disease).
BILIARY TRACT
258
represented the same process prompted a revision in 1977 and this is the one
in use today (Todani et al ., 1977 ).
There have been subsequent classifications (Komi et al ., 1992 ) but these do
not add anything further in the clinical setting as treatment is dictated by the
location of the cyst(s).
Patients with choledochal cysts frequently have associated pathology in the
biliary tract or pancreas including cholecystitis, stones in the bile duct or cysts,
intrahepatic stones or abscesses, pancreatitis, cholangiocarcinoma, and cir-
rhosis with or without portal hypertension (Ono et al ., 1982 ; Nagorney et al .,
1984 ; Ando et al ., 1998 ). The incidence of malignancy in bile duct cysts is
significant and estimations range from 2.5–28% (Fieber and Nance, 1997 ).
Approximately 25% of adults with common duct cysts will present with evi-
dence of cirrhosis or hepatic fibrosis secondary to chronic biliary obstruction
(Nagorney et al ., 1984 ; Soreide et al ., 2004 ). Pancreatitis also occurs in patients
with bile duct cysts and there is a high incidence of malignant change in the
bile duct in these patients (Nagorney et al ., 1984 ).
The incidence of potential complications and particularly the risk of malig-
nant transformation associated with cysts in adults mandate their removal,
although symptoms from stones and sepsis frequently dictate the same course
(Gigot et al ., 1996 ; Nicholl et al ., 2004 ). Extrahepatic choledochal cysts require
a cholecystectomy, mobilization of the cyst, and dissection down into the pan-
creas, sufficient to be below the cyst or as far as safely practical. Some authors
describe being unable to mobilize the posterior wall from the adjacent struc-
tures but careful dissection should enable the entire cyst to be freed and excised.
Proximal anastomosis will be to either the common hepatic duct or by two
individual anastomoses onto the right and left hepatic ducts. If choledochal
cysts are within one lobe of the liver, a resection is required but bilobar disease
has to be managed with extrahepatic cyst resection or by liver transplantation
(Gigot et al ., 1996 ; Chowbey et al ., 2002 ; Lipsett and Pitt, 2003 ; Nicholl et al .
2004 ; Singham, 2007 ).
Caroli’s disease
In Caroli’s disease (Caroli et al ., 1958 ) there are multiple dilatations of the
segmental bile ducts and affected adults may present with a localized abnor-
mality of the liver or with a diffuse process involving the whole biliary tree.
Treatment depends on associated pathology and the presence of complications
such as portal hypertension. The most frequent complication is recurrent
cholangitis and the frequency of infective episodes varies widely from patient
to patient with some experiencing one to two a year and others being disabled
by weekly attacks (Fevery et al ., 1972 ). Intrahepatic stones develop frequently
BENIGN CONDITIONS
259
and can migrate into the bile duct and give obstructive symptoms (Sahel et al .,
1976 ). Like choledochal cysts, recurrent sepsis and biliary obstruction pro-
duces secondary pathology including biliary cirrhosis and cholangiocarcinoma
and there is also an association with hepatic fibrosis (Barros et al ., 1979 ; Dayton
et al ., 1983 ; Witlin et al ., 1982 ).
When the liver is not diffusely involved, segmental resections with bilioen-
teric anastomosis may be useful and good results can be achieved (Nagasue,
1984 ). Transhepatic decompression will improve some of these patients
(Witlin et al ., 1982 ) but when the involvement is widespread, local treatments
are generally ineffective and liver transplantation may have to be considered
(Waechter et al ., 2001 ; Scharschmidt, 1984 ). Overall, however, the results of
the treatment of Caroli’s disease involving both lobes of the liver or associated
with portal hypertension from congenital hepatic fibrosis or secondary biliary
cirrhosis remain poor.
Postsurgical intrahepatic stones
Intrahepatic stones are prevalent in East Asia and are being increasingly recog-
nized in the West due to immigration. They nevertheless remain rare and the
majority of stones that are seen arise secondary to an obstructed biliary system.
This is usually secondary to a stricture which has developed at the site of a
Roux-en-Y anastomosis to the biliary tree but can also occur with chronic
unrecognized obstruction from a stricture following any gallbladder, biliary, or
liver surgery. For any obstruction with proximal stone the management will
depend on the fitness of the patient and the success of minimally invasive tech-
niques. Endoscopic or transhepatic approaches can be used and balloon dilata-
tion is occasionally successful. An endoscopic stent will relieve the obstruction
but not infrequently clinical or biochemical signs of infection continue and
there is a significant long-term risk of biliary cirrhosis. Consequently, many of
these patients eventually come to surgery and this usually requires re-fashion-
ing of the anastomosis and use of a flexible choledochoscope and Fogarty cath-
eter to clear all stones and debris from the intrahepatic bile ducts. Because of
the significant rate of recurrent stenosis and stones (particularly with diathermy
injuries at cholecystectomy) access limbs are very useful in these patients to
provide a potential non-surgical approach. An access limb is simply created by
leaving the afferent end of the jejunal loop used for the bilioenteric anastomosis
significantly longer than normal (of sufficient length to comfortably reach the
abdominal wall). It is then tacked to the peritoneal surface above the incision
so that it can be subsequently accessed percutaneously if necessary. To help the
radiologist locate the blind limb we used to place some ligaclips around the
loop of bowel but now use a sheathed wire sling (see ‘Access limbs’ Fig. 5.13 ).
BILIARY TRACT
260
Dilated bile duct ( Fig. 5.4 )
Idiopathic
Patients whose CBD dilatation is progressive, without evidence of change in
their liver function tests, are a rare but frustrating group of patients. If these
patients are unresponsive to an ERCP and sphincterotomy, continue to dilate
on repeat ultrasounds, but still show no abnormalities of liver function tests, a
biliary bypass is justified, particularly in young patients. If liver function tests
start to demonstrate abnormalities then drainage of the bile duct is mandatory.
Endoscopic stenting will help with some of these patients but with very dilated
bile ducts the stents can block frequently and sometimes do not produce reso-
lution of the liver function test abnormalities. In these patients biliary bypass
is essential to avoid the insidious consequence of obstruction which is biliary
cirrhosis. Many of the idiopathic patients, however, will be shown to have
ampullary stenosis as the underlying cause.
Ampullary stenosis ( Fig. 5.5 )
Ampullary stenosis is probably under-recognized and in many patients with a
dilated bile duct of unknown origin, ERCP will demonstrate a small flat
ampulla which is difficult to cannulate. When cannulated, however, it is usu-
ally obvious that the bile duct is dilated all the way down to the ampulla, the
bile is under pressure, and this is relieved by a sphincterotomy or stent.
These findings are rare in younger patients and care must be taken to exclude
an ampullary tumour. This can normally be done confidently on the appear-
ance but if there is any doubt (in some patients who have previously passed
stones the appearance can be irregular) a biopsy will be needed. Small adeno-
carcinomas can be completely covered by mucosa (Albores-Saavedra et al .,
2000 ) and any bulky ampulla in a patient with biliary obstruction is best treated
by sphincterotomy and biopsy of the submucosal component. In younger
patients, and particularly those less than 30 years, if there is no clinical suspicion
of an ampullary tumour then we prefer to insert a small stent and only proceed
to a formal sphincterotomy if this relieves the symptoms and the bile duct
returns to normal dimensions. If a sphincterotomy fails to produce an improve-
ment in symptoms and liver function tests (usually due to associated pathology
such as pancreatitis) then a formal biliary bypass procedure with a Roux loop
choledochojejunostomy procedure may be necessary (see Section 1).
Pancreatitis ( Fig. 5.6 )
Acute or chronic pancreatitis may lead to biliary strictures and the presenta-
tion particularly with chronic pancreatitis can be insidious. In patients
with chronic pancreatitis it is believed that up to 30% have some degree of
261
DILATED BILE DUCT
Fig. 5.4 Investigation of a dilated common bile duct.
Stones in the
gallbladder
MRCP
EUS
CT
No pancreatic
mass
MRCP
Ampullary
stenosis
Pancreatic mass
(see Section 1)
No stones in the
gallbladder
Dilated CBD on
investigation
No stones in
CBD
No evidence of
pancreatitis but
stricture
Stones in CBD
Evidence of
pancreatitis
Passed stones
Narrow lower
end of the CBD
Narmal LFTs
Jaundiced ERCP
ERCP
Cholangiocarcinoma
(see Section 5)
ERCP
Ampullary
stenosis/tumour
Conservative
treatment
Cholecystectomy
BILIARY TRACT
262
obstruction (Sarles et al ., 1991 ), and while this can be managed temporarily
with balloon dilatation or stenting a number of patients will eventually require
surgery (often at the time of another procedure or definitive treatment). In
these patients a hepaticojejunostomy (see Section 1) is usually very effective
and not infrequently improves their symptoms.
Fig. 5.6 Appearance of the bile duct obstruction in pancreatitis. The duct is smoothly
narrowed in its intrahepatic course and the pancreatic duct is often abnormal.
Fig. 5.5 Appearance of the bile duct in ampullary stenosis. The dilation continues to
the ampulla, the duct is uniformly enlarged, and the pancreatic duct is normal.